Intracardiac Leiomyomatosis:Case Report And A Comprehensive Analysis Of205Cases

Objectives:Intracardiac leiomyomatosis is rare but has been increasingly reported in recent years. We present here a woman with an intracardiac leiomyoma originating from uterine leiomyomatosis. The tumor was completely removed in a one-stage procedure. We also provide a detailed and comprehensive review of the etiology, epidemiology, clinical presentation, diagnosis/differential diagnosis, histopathological characterization, treatment and prognosis of this disorder.Methods:We present here an intracardiac leiomyoma which was completely removed in a one-stage procedure. We performed an electronic literature search for intracardiac leiomyomatosis on MEDLINE/PubMed, Web of Science and Cochrane Library. Case reports and case series published in English with final diagnosis of intracardiac leiomyomatosis confirmed by histopathological examination were included. All the references cited in each publication were evaluated for additional eligible cases.Results&Conclusions:Finally, we identified205cases of intracardiac leiomyomatosis that were reported in English up to April2013. According to our collection, intracardiac leiomyomatosis is seen exclusively in women. Age prevalence giving an average47.3years and it is most common in the fifth decade. Most patients had undergone a previous hysterectomy/myomectomy or had a coexisting uterine leiomyoma. Symptomatic patients presented with a various symptoms, the most common being dyspnea, syncope, edema of the lower extremities and palpitation. Transesophageal echocardiography, CT and MRI are helpful to the preoperative diagnosis and guide the surgical management. When a right atrial mass is identified in middle-aged women with previous hysterectomy/myomectomy or uterine leiomyoma, a high degree of suspicion of intracardiac leiomyomatosis should be maintained and a thoracic/abdominal CT should be arranged to confirm the origin. Complete removal guarantees an excellent outcome with no recurrence or postoperative death, while incomplete removal leads to recurrence in one-third of patients. Anti-estrogen therapy is not imperative after incomplete removal due to its inability to prevent recurrence.